Pediatric Urology

5/2/2012
Pediatric Urologic Problems:
A General Practitioner Perspective
Ali Ziada
Pediatric Urology - University of Kentucky
Pediatric Urology
• New Specialty being recognized.
• Europe
• 2006: UEMS recognized new specialty
• New Board Created
• 2008: First FEAPU exam held
• USA:
• Subspecialty certification
• 2009 First Pediatric Urology Boards
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5/2/2012
San Francisco 27-31 May 2010
Pediatric Urology Scope
• Circumcision
• Hypospadias
• Scrotal pathology
• Antenatal hydronephrosis
• UTI & Vesicoureteral reflux
• Urinary obstruction: UPJO & UVJO
• Neurogenic bladder & Incontinence
• Stone disease
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Phimosis & Circumcision
Circumcision
• No absolute medical indication in the neonatal
period
• Relative indication: recurrent infections
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Foreskin Care
• By 3 years of age 90% of boys have a retractable foreskin
• Suggest to parents
• Normal cleaning
• No forceful retraction
• Teach boys to pull back foreskin to void
• Treatment only necessary for phimosis causing infection or
difficulty voiding
Circumcision
• Potential medical advantages
• Decrease incidence of UTI in the first year of life
• Prevent phimosis
• Prevent balanoposthitis
• Decrease incidence of penile cancer
• May decrease the incidence of sexually transmitted disease
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5/2/2012
Complications
• Rare 0.2-0.5%
• Bleeding
• Injury to penis
• Skin issues
• Take off too much/Leave on too much
• Skin bridges
• Inclusion cysts
• Penile curvature
• Urethrocutaneous fistula
• Meatal stenosis
Complications hidden penis
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5/2/2012
Circumcision methods
• Gomco clamp
• Plastibell clamp
• Mogen
M
clamp
l
• Surgically
Gomco Clamp
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Plastibell
Mogen Clamp
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Phimosis & Paraphimosis
Phimosis
• Narrowing of the opening of the
prepuce
• Treatment
• Steroid cream (0.05%
Betamethasone cream))
• Circumcision
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5/2/2012
Paraphimosis
• Phimotic ring of foreskin is retracted by
th child
the
hild or parentt
•
trapped proximal to coronal sulcus
•
significant glans edema
•
possible ischemic injury
• Treatment involves
• Manual reduction of the foreskin
• Surgically by division of the phimotic ring
• Circumcision is advisable
Paraphimosis
• Painful constriction of the glans penis by the foreskin which
has been retracted behind the corona
• Treatment: dorsal slit
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5/2/2012
Balanitis
• Nonspecific etiology; inadequate hygiene or external irritation
• Balanitis (inflammation of the glans penis)
• Posthitis (inflammation of the foreskin)
• Balanoposthitis (inflammation of the glans and the foreskin)
• Inability to retract the foreskin and accumulation of smegma
• In severe cases, when the foreskin cannot be easily retracted, oral
antibiotics may be required
Hair torniquet
• A surgical
i l emergency
similar to paraphimosis
in appearance and
pathophysiology.
• Ischemic
sc e c injury
ju y to
o the
e
glans may occur if not
relieved promptly by
division and removal of
the hair strand.
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Hypospadias
Hypospadias
• Second most common birth
defect, and
• Incidence increasing.
• Etiology unknown
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Hypospadias
Male human fetal external
genitalia during gestation
At 11 weeks, the urethra is open and urethral fold
(uf) and groove are prominent in the
transillumination view of the phallus.
At 16.5 weeks, normal ventral curvature (vc) is seen
and the foreskin is almost complete.
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5/2/2012
•(C) At 20 weeks penis and urethra are complete
with penile curvature resolved.
(D) At 24 weeks prepuce covers whole glans
ms: the midline seam
TIP
(Snodgrass)
Repair
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TIP repair
Proximal Hypospadias
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Preputial or Buccal Mucosa Grafts
Hypospadias- associated
abnormalities
• Easy to remember - nothing!
• Normal kidneys and bladder
• Normal fertility
• Normal sexual function
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5/2/2012
Hypospadias - management
•For
pediatricians
• Do not circumcise ??
• No need for imaging
• Refer to pediatric
urologist
• Consider DSD if
associated with
undescended testis
For Pediatric Urologists
• Surgery at 6 - 9 months
• Attempt one stage
reconstruction
• Outpatient surgery
• Success rates 95%
Who is a Boy?
& who is A
Girl?
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5/2/2012
Exstrophy Epispadias
Epispadias
•Very
rare
•More
often associated with
bladder exstrophy
•Need
earlyy referral for p
parental
counseling
•Patients
may be totally
incontinent
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5/2/2012
Female epispadias
•
Rare condition
•
In females this condition is
rare, at ≈1/480 000
•
Less severe variants can
result in sphincter
incompetence without
obvious clinical signs.
•
History of chronic wetting
without a diagnosis realized.
Incidence
• 1 in 50,000 live births
• Incontinent epispadias alone 1
in 100,000 live births
• Male to female ratio 2:1
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5/2/2012
Anatomy Classic type
• Wide symphysis pubis.
• Outward rotation & shortening of
pubic rami.
• Waddling gait in early childhood
• Few cases have hip dislocation
and spinal defects
Abdominal & Pelvic defects
• Abdominal Wall Defects
• Short distance between umbilicus and anus.
• Umbilical hernia usually present and of insignificant size.
• Frequent indirect inguinal hernias.
• Pelvic Floor Defects
• Levator ani is more posterior in exstrophy.
• The levators are rotated outward and more flattened.
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5/2/2012
Male
Genital defects
Anterior corporal length 50% shorter.
Urethra anterior to the prostate.
Female
Clitoris is bifid
Vagina is shorter but normal caliber.
Vaginal orifice is stenotic and anterior
Prenatal Diagnosis & Management
• Absence of bladder filling,
• A low-set umbilicus,
• Widening pubis ramus,
• Diminutive genitalia
genitalia, and
• A lower abdominal mass.
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5/2/2012
Posterior urethral valve
PUV
• Most common structural cause of
urinary outflow obstruction in
pediatric practice
• Most common obstructive uropathy
leading to childhood renal failure
• High incidence of renal failure in
patients prenatally diagnosed
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5/2/2012
valve resection
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5/2/2012
Pathological effects of
urethral valves
• Hydroureteronephrosis
• Vesicoureteral reflux,
• Detrusor dysfunction.
• Abnormal
Ab
l renall ttubular
b l
function
• Reduced glomerular
filtration
Valve bladder
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5/2/2012
Acute Scrotum
Scrotal emergencies
• Common
• Urgent
• Scrotal
S t l swelling
lli + pain/erythema
i / th
• r/o torsion
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5/2/2012
Differential diagnosis
•
Testicular torsion
•
Torsion testicular/epididymal appendage
•
Epididymitis
•
Incarcerated hernia
•
Trauma
•
Insect bite
Keys to diagnosis
• Age of child
• History
• P/E
• Timing of presentation
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Testicular torsion
• Bimodal age presentation
– Perinatal period Extravaginal torsion
– Any age (puberty peak) Intravaginal
 Intravaginal torsion
Testicular torsion
• Testicular torsion requires emergent surgery
• Late exploration
p
not usually
y indicated
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5/2/2012
Extravaginal torsion
• Management of extravaginal torsion (controversial)
– No intervention
– Urgent exploration
–
excise necrotic testis & fix contralateral
– Semielective exploration
–
optimize anesthetic risk
Intravaginal torsion
• Abnormal fixation of testis within tunica vaginalis
g
• Patients present with acute pain
• Later hemiscrotum becomes erythematous
• Transverse lie early
• Cremasteric reflex classically absent
• No imaging needed before emergent surgery.
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5/2/2012
Testicular torsion
Intermittent torsion
• Prior similar episodes
• Spontaneous ‘‘detorsion.’’
• Subsequent
S b
t testicular
t ti l torsion.
t i
• Elective fixation
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Differential
•
Torsion of appendix testis
• Point of tenderness localized
• Usually upper pole
• Blue dot sign
• Self-limited
•
Epididymitis
p
y
or orchitis
• Hx dysuria & fever or instrumentation
• Voiding symptoms + bacteriuria
• Pre- and postpubertal boys.
Testicular salvage
• Salvage directly related to duration
– Excellent salvage expected if <6 hours
– Progressively worse rates thereafter.
– Beyond 48 hours,
hours salvage rate is poor
• Delay presentation >>> diagnosis becomes
obscure.
• Urinalysis, WBC, and ultrasonography
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5/2/2012
Imaging
• Boys who have an acute scrotum DO NOT require an
ultrasound.
• H&P consistent with torsion  immediate surgery
• Doppler
D
l US is
i technician
t h i i dependent
d
d t and
d NOT 100%
sensitive or specific for torsion
• In equivocal cases, it can be helpful
Scrotal trauma
•
A di
directt bl
blow or straddle
t ddl iinjury
j
•
Hematoma, ecchymosis or rupture
•
The examination can be difficult
•
Doppler ultrasound can be a useful adjunct.
•
Testicular rupture can be present despite an ultrasound
showing an intact tunica albuginea.
•
If the testicle is not intact, urgent surgical repair.
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5/2/2012
Incarcerated inguinal hernia
•
Tender or nontender
scrotal swelling
•
Persistent mass and
induration extending to the
inguinal area
•
Prior suggestive history
•
Manual reduction can
delay surgical exploration
•
Surgical exploration if
reduction is not possible.
Hydroceles & spermatoceles
• Nontender scrotal swelling
• Transilluminate.
• Lack of persistent mass in the upper scrotum and
external inguinal ring along the spermatic cord.
• Noncommunicating hydroceles can be electively
corrected after the age of 2 years
• Spermatoceles do not require excision unless desired by
the patient
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5/2/2012
Varicoceles
•
15% of boys
•
Present as a scrotal swelling
or discomfort
•
Examination standing and
supine with & without
Valsalva
•
Management controversial
•
May be a sign of
retroperitoneal process
Undescended testis
• 3-6% of all newborn
• 1% of all 1-year-old boys
• Genetic inheritance of this disease is unclear.
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5/2/2012
Undescended testis
•Palpable
or nonpalpable?
•Unilateral
or bilateral?
•Associated
hypospadias?
•Associated
syndromes?
•Most
will have isolated unilateral undescended
testis
Undescended testis
•Intraabdominal
•testis
(nonpalpable)
located above internal ring
•Canalicular
C
li l
•Retractile
“ ti ” undescended
“routine”
d
d d ttestis
ti
- not a UDT
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5/2/2012
Undescended testis
•Observation
•If
until 6 -12 months of age
still undescended, surgical correction
•No
advantage to further observation after 12 months
of age
•testis
will not descend
•germ
cell fibrosis evident by three years of life
Genital abnormalities
•Genital abnormalities requiring urgent evaluation include ambiguous
genitalia, hypospadias with nonpalpable testis & interlabial masses.
•Ambiguous genitalia often presents a social emergency and a potential
medical emergency, owing to potential life-threatening, salt-wasting CAH.
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5/2/2012
Ambiguous genitalia
• Evaluation includes karyotype,
karyotype electrolytes,
electrolytes 17
17-OH
OH
progesterone, testosterone, LH, and FSH in the immediate
perinatal period.
• Possible presentations include clitoral enlargement with a
palpable labioscrotal mass, microphallus with hypospadias,
and clitoromegaly with labial fusion.
• If the 17-OH progesterone level is elevated  CAH
• If the 17-OH progesterone level is normal  further
endocrine evaluation
Interlabial masses
Gartner’s duct cystImperforate hymenUrethral prolapseProlapse ureterocele
Normal urethral
meatus
Smooth
translucent lesion
protruding from or
filling the vagina.
Asymptomatic
Drain
t
l
Shiny bulge over
vaginal introitus.
Urethral meatus
uninvolved
Everted urethral
mucosa.
Treatment includes
observation,
topical antibiotic,
estrogen creams &
sitz baths Rare
surgical excision
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Voiding dysfunction
Bladder function
•Store
urine at low pressure
•Empty
urine
•Bladder
is in storage mode for 23 hours and 45
minutes
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5/2/2012
Voiding Dysfunction
History
•Daytime
Investigations
vs. nighttime wetting vs. both
•Screening
•Ensure
•Urgency?
•Frequency?
•Damp
p
•Does
•Bowel
wall thickness
•Post-void
vs. soaking?
g
function?
normal kidneys
•Bladder
Infrequent voiding?
child care if wet?
ultrasound
residual
•Functional bladder capacity by
voiding diary
•VCUG
Differential diagnosis
•Bladder
instability/overactivity
•Infrequent
voiding
•Incomplete
•Hinman’s
emptying
syndrome
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5/2/2012
Voiding Dysfunction
Treatment
•Anticholinergics
•Timed
voiding
•Intermittent
•Bowel
catheterization
management
•Prophylactic
antibiotics
Enuresis
• Prevalence 15-20% of 5 year olds & 1-2% adolescents
• Cause: Genetic, maturational delay, difficulty awakening,
psychological
• Investigation: urine and nothing further if only nocturnal
• Treatment
• Reassurance
• Bed alarm
• DDAVP + Anticholinergics
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5/2/2012
Hydronephrosis
Antenatal Hydronephrosis
• Incidence 1/100
pregnancies
• Only
O l 1/500 are significant
i ifi
t
abnormalities
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5/2/2012
Differential Diagnosis
• Normal
• Uretero-Pelvic Junction Obstruction
• Vesicoureteral reflux
• Ureterovesical junction obstruction (megaureter)
• Multicystic Dysplastic Kidney
• Posterior urethral valves
• Prune Belly syndrome
Investigation
• Immediate postnatal ultrasound if
• Bilateral severe hydronephrosis
• Dilated posterior urethra suggesting valves
• Repeat ultrasound 4-6 weeks and if persistent
• VCUG
• MAG3 renal scan
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5/2/2012
SFU classification
• Grade 0
no dilatation
• Grade I
only pelvic dilatation
• Grade II
pelvis + calyces visible
• Grade III
pelvis + calyces dilated
• Grade IV
parenchymal thinning
• Calyceal dilatation is not an indication for early
delivery
Renal pelvic diameter
• A-P diameter of the renal pelvis
• 10-20 mm
mild , probably reversible
• 20-30
0 30 mm
moderate,
ode ate, equ
equivocal
oca
• > 30 mm
severe, probably will need postnatal
intervention
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5/2/2012
Urinary Obstruction
UPJ
Megaureter
Ureteropelvic Junction
Obstruction
• Congenital stricture or adynamic
segment
• Crossing vessel
• Ultrasound & MAG3 scan
• Asymptomatic >> Observe
• Worsening renal function or hydro
operate
• Symptomatic >> Operate
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5/2/2012
Goal
• Relief obstruction
• Preservation
P
ti or iimprovementt off function
f
ti
Indications
• Symptomatic obstruction
• Impairment of overall renal function
• Progressive impairment of ipsilateral renal
function
• Development of stones, infection
• Hypertension
• Timing of repair is controversial
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5/2/2012
Pyeloplas
ty
• Stood the test of time
• 98% success
• Provides widely patent,
dependently positioned well
funneled UPJ
• Variety of incisions
• Variety of techniques
Ureterovesical Junction Obstruction
“Megaureter”
• Obstruction or adynamic segment at the UVJ
• Presentation: Often antenatal ultrasound
• Investigation: Ultrasound + MAG3 renal scan
• Management
• Observe if function preserved
• If function deteriorating correct surgically
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5/2/2012
Vesicoureteral Reflux
• Abnormal retrograde flow of
bladder urine back into the
upper urinary tract
• Sibling predisposition: 40%
• Presentation
• Antenatal ultrasound with
hydronephrosis
• Urinary tract infection
Grades of VUR
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Endoscopic VUR treatment
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5/2/2012
Surgical treatment of Reflux
Collecting System
Abnormalities
Partial
Duplication
Complete
Duplication
Intravesical
Ureterocele
Ectopic
Ureterocele
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Weigert-meyer rule
Ureterocele
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5/2/2012
Single system ureterocele
Duplex system Ureterocele
A.intravesical ureterocele entirely within the bladder.
B.distal portion of an ectopic ureterocele extends outside the bladder
Ureterocele
Ureteral
duplication
•Reflux
into both
ureters of a complete
duplication is less
common than reflux
f
into the lower pole
ureter alone
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5/2/2012
Ectopic Ureter
i M
in
Males
l &
Females
Surgical options
• Upper pole surgery (open vs lap)
• partial nephrectomy
• pyelo-ureterostomy
• Ureteral reimplantation
p
((and ureterocele excision))
• Combined upper and lower tract approaches
• Lower tract uretero-ureterostomy (U-U)
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5/2/2012
Surgical techniques
•Excision
of ectopic ureterocele &
common sheath reimplantation
Upper pole nephrectomy
UTI: Presentation
• Young children
• Fever
• Decreased appetite
• Lethargy
• Older children
• Fever (implies pyelonephritis)
• Dysuria
• Frequency
• Abdominal pain
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5/2/2012
UTI: investigation
•H&P
• Voiding
g history
y
• Family history
• Fever
• Urine: Ideally cath specimen; bag specimens are
bad
• Radiology
• U/S
• VCUG
• DMSA
UTI Treatment
• Lower tract: short course
antibiotic
• Upper tract (fever, back pain,
nausea and vomiting): 2
week
k course, admission
d i i if
very ill
• Quick treatment decreases
chances of scarring
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5/2/2012
Thank you
53